A family with Peutz-Jeghers syndrome and bilateral breast cancer.

The proband and her paternal grandmother had bilateral breast carcinoma and the Peutz-Jeghers syndrome. An ovarian sex cord tumor with annular tubules was an incidental finding when oophorectomy was performed as treatment of the proband's premenopausal breast cancer. Because there are previous reports of breast cancer in patients with this syndrome, the Peutz-Jeghers gene may be associated with an increased risk of breast tumors.